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Postgraduate Medical Journal 2001;77:717-722; doi:10.1136/pmj.77.913.717
© 2001 BMJ Publishing Group Ltd and The Fellowship of Postgraduate Medicine.
Postgrad Med J 2001;77:717-722 ( November )

Case reports

Non-hepatic hyperammonaemia: an important, potentially reversible cause of encephalopathy N D Hawkesa, G A O Thomasa, A Jurewiczb, O M Williamsc, C E M Hillierc, I N F McQueenc, G Shortlandd

a University Hospital of Wales: Department of Gastroenterology, b Department of Surgery, c Department of Neurology, d Department of Paediatrics

Correspondence to: Dr N D Hawkes, Department of Gastroenterology, University Hospital of Wales, Heath Park, Cardiff CF14 4XN, UK neilhawkes{at}doctors.org.uk

Submitted 21 August 2000; Accepted 15 February 2001

The clinical syndrome of encephalopathy is most often encountered in the context of decompensated liver disease and the diagnosis is usually clear cut. Non-hepatic causes of encephalopathy are rarer and tend to present to a wide range of medical specialties with variable and episodic symptoms. Delay can result in the development of potentially life threatening complications, such as seizures and coma.
Early recognition is vital. A history of similar episodes or clinical risk factors and early assessment of blood ammonia levels help establish the diagnosis. In addition to adequate supportive care, investigation of the underlying cause of the hyperammonaemia is essential and its reversal, where possible, will often result in complete recovery. Detection of an unborn error of metabolism should lead to the initiation of appropriate maintenance therapy and genetic counselling.


Keywords: hyperammonaemia; encephalopathy; portosystemic venous shunt; urea cycle defect

© 2001 by The Fellowship of Postgraduate Medicine
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